Preservation of the Bladder in Patients With Rhabdomyosarcoma

Purpose: To review the pathologic findings from children with gross residual rhabdomyosarcoma (RMS) of the bladder and compare the treatment outcome of those who underwent cystectomy with those who did not. Patients and Methods: Primary and follow-up records and pathology specimens for 28 patients with gross residual disease entered onto the Intergroup Rhabdomyosarcoma Study (IRS) III were reviewed. These patients were assigned to receive 20 weeks of multiagent induction chemotherapy and 4 weeks of radiotherapy. Future therapy decisions were based on clinical and histologic evaluation at 20 weeks. Results: All patients had a clinical and histologic response. Thirteen patients underwent cystectomy at intervals that ranged from 1.5 to 38 months after the start of therapy. All but one patient are alive and well without recurrence. Reasons for cystectomy included presumed evidence of tumor growth from imaging studies, findings at cystoscopy, or histologic interpretation of biopsies. In HE GOAL OF THE Intergroup Rhabdomyosarcoma Study (IRS) for patients with primary bladder or bladder/prostate rhabdomyosarcoma (RMS) is not only survival, but survival with an intact and functioning bladder.3 Retention of the bladder may also minimize some of the long-term problems of sexual dysfunction associated with cystectomy. The use of partial cystectomy has been fostered to attain this goal, but is applicable in a relatively limited number of cases. 4 5 Among patients in IRS III with gross residual disease after biopsy, subsequent cystectomies were performed most frequently in patients with intravesical primary tumors (43%), less often in those with prostatic primary tumors (36%), and least often in those with extravesical primary tumors that did not extend through the bladder wall (14%). Because more cystectomies were performed for intravesical primary tumors and subsequent biopsies more readily obtained at cystoscopy, this group was chosen for review. Pathologic review of primary and follow-up specimens was made, comparing findings between those patients who retained the bladder and those who underwent cystectomy. PATIENTS AND METHODS Patient Selection One hundred three patients were entered onto IRS III with primary tumors that involved the bladder between November 1984 and September 1988. There were 35 patients with positive biopsies from intravesical bladder sites, 24 with abdominal or pelvic masses with bladder attachment, and 44 with prostatic or bladder/prostate involvement. Among the 35 intravesical tumors, four were group I cases (no gross or microscopic residual disease after initial surgery) and all are living and well following partial cystectomy and chemotherapy. A single group II patient (microscopic residual disease) underwent cystectomy as primary therapy for an intravesical tumor that extended through the bladder wall to an abdominal mass. This patient died of pneumonia after 8 months of chemotherapy and radiotherapy. Two of 30 patients with group III disease died following 2 and 3 weeks of therapy from toxicity related to chemotherapy. The remaining 28 group III patients comprise the current study. Treatment After initial biopsy, patients with gross residual disease (group III) were scheduled to be treated with 20 weeks of induction chemo

Results of treatment of patients with superficial facial rhabdomyosarcomas on protocols of the Intergroup Rhabdomyosarcoma Study Group (IRSG), 1984-1997

PURPOSE: We analyzed the outcome of 47 patients with superficial facial rhabdomyosarcoma (RMS) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols-III, -IV-Pilot, and -IV. METHODS: We reviewed patients\u27 records. Clinico-pathologic features, treatment, and outcome were examined to identify prognostic factors. RESULTS: Thirty-two patients were males; 35 patients were 1-9 years old at diagnosis. Tumor sites were buccal/cheek (N = 21), external nasal/nasolabial (N = 12), lip/chin (N = 9), and masseter (N = 5). Patients (46/47) had localized disease: 18 biopsy only (Group III), 17 microscopic residual tumor (Group II), and 11 complete resection without residual tumor (Group I). Eight-year estimated event-free survival (EFS) and overall survival (OAS) rates were 61% and 65%. Patients EFS, 21%, compared to approximately 68% in older patients (P = 0.077). Eight-year EFS rates were 80% for females and 50% for males (P = 0.096). Eight-year EFS rates were 72% in 33 patients without regional lymph-nodal tumor and 39% in 14 patients with regional nodal tumor (P = 0.07). Eight-year EFS rates were 72% for 22 patients with embryonal RMS and 53% for 23 patients with alveolar RMS (P = 0.28). Location of the primary tumor was not significantly related to outcome. CONCLUSIONS: Patients with superficial facial RMS often have localized, grossly resectable lesions at the time of presentation. Favorable prognostic factors include age \u3e12 months, female gender, embryonal histology, and no lymph-nodal tumor

Outcome of patients with localized orbital sarcoma who relapsed following treatment on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols-III and -IV, 1984-1997: a report from the Children\u27s Oncology Group

BACKGROUND: We wanted to ascertain patterns of recurrence, re-treatment, and outcome among 188 eligible patients treated for localized orbital sarcoma on IRSG Protocols III/IV, 1984-1997. PROCEDURE: Retrospective chart review. RESULTS: Twenty-four of 188 patients (12.8%) developed local (n = 22) or distant relapse (n = 2) at 0.057-7.05 years (median, 1.58) after enrollment. Ages at study entry were 0.14-17 years (median, 5 years). Initial tumor operations included biopsy (n = 20) or gross resection with microscopic residual (n = 4). Initial tumor diameters were 0.5-7 cm (median, 3). Pathologic subtypes were embryonal rhabdomyosarcoma (ERMS, n = 19), sarcoma not otherwise specified (n = 2), and alveolar RMS, botryoid ERMS, or undifferentiated sarcoma (n = 1 each). Initial treatment included vincristine/dactinomycin (n = 24) including an alkylator (n = 4) and radiotherapy (RT, n = 21). Twenty patients responded, 14 completely, 6 partially. After recurrence, patients underwent orbital exenteration (n = 10), enucleation (2), tumor excision (3), or biopsy (1); 7 had no operation, and 1 had no data. Post-relapse chemotherapy included combinations of etoposide (n = 14 patients), doxorubicin (14), ifosfamide (12), cyclophosphamide (7), and dacarbazine (n = 1). Six patients received RT, including four previously treated and two not irradiated initially. Two patients died; one at 1.79 years after contralateral brain metastasis followed by local recurrence, and another at 2.49 years after multiple local recurrences. Twenty-two patients (91.7%) survived sarcoma-free for 0.04-17 years (median, 6.9) after relapse, and 18 of 22 (82%) were alive \u3e/=5 years after relapse. CONCLUSION: Survival following recurrent localized orbital sarcoma appears likely after vigorous re-treatment given with curative intent

The Intergroup Rhabdomyosarcoma Study Group (IRSG): Major Lessons From the IRS-I Through IRS-IV Studies as Background for the Current IRS-V Treatment Protocols

Purpose. To enumerate lessons from studying 4292 patients with rhabdomyosarcoma (RMS) in the Intergroup Rhabdomyosarcoma Study Group (IRSG, 1972–1997)

Results of treatment of fifty-six patients with localized retroperitoneal and pelvic rhabdomyosarcoma: a report from The Intergroup Rhabdomyosarcoma Study-IV, 1991-1997

BACKGROUND: We reviewed 56 IRS-IV patients with localized rhabdomyosarcoma [RMS] of the retroperitoneum/pelvis to assess outcome and prognostic factors, including the value of initially excising \u3e or=50% of the tumor (debulking) before chemotherapy. METHODS: Patients had embryonal RMS [N=38], alveolar RMS [N = 7], RMS not otherwise specified [NOS, N = 7], or undifferentiated sarcoma [N = 4]. Fifteen patients were debulked; 41 patients were biopsied. All received VAC; most received radiotherapy. RESULTS: Estimated 5-year failure-free survival [FFS] and overall survival rates were 70 and 75%, respectively. FFS rates were better for patients /undifferentiated sarcoma. After adjusting for age and histological differences, FFS was better for patients whose tumor was debulked prior to beginning therapy [P = 0.02]. CONCLUSIONS: These results are superior to those of previous protocols for patients with RMS of the retroperitoneum/pelvis. Initial excision of \u3e or=50% of the tumor may be associated with increased FFS